It Came to Pass

The scriptures say “And it came to pass…” I love that.

That phrase is sprinkled liberally throughout the Book. And I believe it. It didn’t come to stay. Most things don’t. I looked forward to the time when I wouldn’t have to get up multiple times at night to feed Celia. I looked forward to sleep. And then it passed and I wished, sometimes, for it to come back, for the chance to nurse her while the rest of the world slept. And I anticipated the terrible twos, knowing they might challenge us, but knowing that stage, too, would pass. But Batten Disease… it’s here to stay. Golly, I bet Batten’s mom was proud of him, identifying a disease and all. I used to tell my fifth graders they were all scientists. I believed it. They did, too. Perhaps someday one of them will identify a disease. Gosh, I’d be proud. Perhaps one of them will discover a cure – even prouder. Perhaps, someday, Batten Disease will come to pass.




We've been busy. Working around the clock. Working till the hands fall off the clock, or the clock melts or something like that. But it's not the long hand or the short one that matter. It's these.

His hands aren't storied yet. They haven't shaped or scrawled or spoken, but they have grasped. And we don't want to let go.



So Happy

Together. All 4 of us. Or 5, counting the dog. Going on 36 hours or so now. There's been some crying, from everyone at some point or another, but the dog has only joined in howling once. And there've been lots of smiles and snuggles. Even some sleep. And, bonus, a load of laundry or two, washed, dried, folded and put away. All without coffee. Or wine. Yet.
The small people do not outnumber the big people. The big people actually sat down and enjoyed dinner together.
We can handle this. We will handle this.

No matter how they toss the dice...


We are all more than we know

We are overwhelmed by the outpouring of support from people. Not just friends and family but friends of friends and colleagues of colleagues... random people that find the blog... it is all very touching.

We seem to have struck a chord in humanity. The impending death of a child, the suffering of a family, the tragedy of the situation, has a universal effect. Many correspondences and conversations point at our strength, our ability to soldier on... The truth is we get up every day (and a lot at night, too) and do what needs to be done. All of you would too, if you had to. We aren't extra special, and there is nothing wonderful or inspiring in what we do - we just do what has to be done and find joy in things a lot of parents take for granted.

One of Jenni's friends gave us a book a while back - Kitchen Table Wisdom: Stories that Heal, by Rachel Remen. It's a nice collection of short stories, the kind of book that is meant to inspire or to invoke reflection on personal situations. One short story in particular stuck with me, and I think describes the Betz Family Columbus pretty well.

The setting for the story is an inner city hospital where a young gang member's wife is dying of cancer, and Rachel, as the chief resident, had a young psychiatry intern shadowing. In her book she retells the story, waxing poetically about the tattoo covered gangbanger's expressions of love and commitment to his dying wife. During the encounter, the shadowing Freudian intern stopped taking notes and his eyes welled with tears. As they left the room, Rachel asked the intern what he learned from the interaction. "We are all more than we seem" he said... to which Rachel wisely wrote "We are all more than we know."

Where in the parenting handbook does it teach us how to deal with tragedy and loss? (Jenni can't even find the chapter on changing little boy diapers without getting peed on!) We have been forced to live in ways we never expected to live, but have discovered unsuspected capacities to forge ahead, and to live nonetheless. We have, in some ways, found more of ourselves.
And we are not alone. Plenty of friends and family and friends of friends and colleagues have been called upon for guidance and direction and comfort. To them, to you, we say "Thank you. You are more than you know."

We are all more than we know.

I wish I were only half the man my dog thinks I am though...



Hope Springs

Edited: the post, below, reflects our gardening ignorance. The entry was written before it occurred to us to do a quick Google fact check, at which point we learned that what we thought was Grandma Eleanor's yellow primrose is actually a yellow winter aconite. Whatever. We're still gonna go with the message we thought the flower was delivering.
Surely you can forgive our amateur gardening skills and still appreciate the point we thought we were making...

Plus, that same quick Google search said something about bright aconite petals, juxtaposed with barren earth or snow, making for a potent visual metaphor - a tough little sprig of a plant overcoming winter, life overcoming the threat of death. Maybe our mistake was meant to lead us to that idea as well...

We've tried to transplant Grandma Eleanor's yellow primrose several times. We've divided plants and taken starts from Mom's yard, and Jan's yard, and we've introduced the cuttings in various parts of our own garden. Never has one survived.
Several days ago, on the very day we received some long-hoped for good news, Andy and I both, upon separate passings, noticed this tiny primrose peeking through the leaves overflowing from our compost pile.
And it was like Grandma Eleanor was sending us a message:
It's spring now, and hope has sprung.
And we heard her, loud and... well, not clear but bright yellow...

Celia Eleanor, sporting Grandma Eleanor's primrose behind her ear (Spring 2008)




We've been accused, lovingly, of blogging cryptically. Maybe we haven't shared the whole truth. We certainly don't intend to sound whiny or to elicit sympathy, and perhaps that's why we avoid some factual postings. But we do realize, for those readers who don't get to see Celia, our entries may be misleading. Allow us to share more specific details, and feel free to ask pointed questions. If you're reading our blog, we imagine you love Celia, are praying for her comfort, and hold our family in your thoughts. You deserve information.
The teacher in me wants to state things positively, like "Please walk" instead of "Don't run," and to focus on what Celia can do rather than dwell on what she can't. In an effort to be honest, I'm resisting that urge here...

By appearances, Celia is not a normally developing two year old. She drools. A lot. And she waves her arms around randomly, has trouble focusing her eyes on things, bobs her head.
She doesn't walk, doesn't stand, doesn't crawl, and has difficulty sitting unsupported.
She doesn't have much purposeful movement; she can't grasp a toy or take your hand or feed herself or put her pacifier in.
She doesn't talk, and when she does make noise it often sounds like she's frustrated or sad.
She often seems irritable or agitated. Her body is stiff, which makes things like getting dressed stressful for her.
She aspirates on thin liquids, so must drink things that have been artificially thickened. We have to mince her food, because she does not chew. We now have a suction machine at home.
She takes multiple medications - 5 mLs of this, 8ccs of that, a quarter tablet of something else to relax, a pill for pain... and we have "emergency meds" in the cabinet were she to have a really scary seizure.
She is now under the care of Children's Hospice.
She still has trouble sleeping, so someone sleeps with her. On average she might go to bed at 10:30,wake several times through the night, and wake for the day at 9ish. She's fairly lethargic during the day. Often she's happy to rest in a bean bag or on the couch, but she prefers to rest on someone.
She doesn't see very well. We suspect she knows people close to her her by their voice and scent.
She is like a 30 pound infant...

Please don't misinterpret this list as us complaining. We try not to complain, try to enjoy our time with Celia. Most days, enjoying her is effortless. She's pretty enjoyable. Like the rest of us, Cel has good days and not so good days... Caring for her can be challenging, but we're up to it. Watching her regress, thinking about what could've been, is depressing, but we can handle it. And for those of you reading, your interest and support and love help us more than you may know.Sometimes we hesitate to post pictures of Celia, unless she looks "normal." It's hard to capture a "good" photo of her these days. Even though she's still stunningly beautiful....



Luck of the Irish

Have you ever felt like there was a four-leaf clover right where you looked for it, like you were surrounded by pots of gold? We have.



Answered Prayers

We've been expecting miracles, and Tucker's birth counts as one. We received news today that his PPT/TTP enzyme levels fall within the normal range, meaning he does not have Batten Disease. Certainly another miracle... Our family is rejoicing!A&J


Celia's Story

Born March 7, 2007, Celia appeared to be a healthy little girl – she developed normally beyond her first birthday, waving and saying “hi” and learning to use a fork and taking steps… but we noticed two years ago now, when Cel was about 15 months old, that her skills had plateaued, and that she was regressing. She stopped using any of the words she’d acquired; eventually she stopped trying to walk. Developmental Specialists at Nationwide Children’s Hospital ordered an MRI and found abnormalities in her brain, but no one could tell us why. Celia stopped sleeping well about that same time, waking 20 times or more each night. An EEG revealed myoclonic seizures (small single jerks, that we weren’t able to notice) were likely waking her. Celia lost the ability to feed herself and her vision became limited. We visited lots of specialists and Celia’s neurologist ordered lots of tests; after months of searching for a diagnosis, it was determined in January 2009, shortly before her second birthday, that Celia has NCL1 -- infantile neuronal ceriod lipofuscinosis, an autosomal recessive disorder more commonly known as Batten Disease. Afflicted children have an enzyme deficiency that causes neurodegeneration. Batten Disease is rare, about 2 in 100,000, and there are only about 150 children living with it in the US. Sadly, families may have more than one affected child. At this time NCL is always fatal, so we were relieved to learn that Celia’s little brother, Tucker, does not have Batten Disease. Children with the disease suffer mental impairment, worsening seizures, and progressive loss of vision and motor skills; most do not survive beyond their first decade. 
Celia takes lots of medicine, her food has to be pureed, and she is no longer able to drink even thickened liquids.  She used to enjoy Baby Einstein DVDs and Justin Roberts’ music, but those things don’t calm her the way they once did.  She still enjoys an occasional stroller ride around our neighborhood, and a dip in her grandparent’s hot tub once in awhile, but her favorite thing to do is to snuggle on someone’s lap and rest.  She lacks any purposeful movement, her fight to crawl now a distant memory, although we enjoy watching her stretch and yawn when she wakes up.  She sleeps much of the day, but she smiles a lot more, too. We cherish her, and the time we have left.
Jenni & Andy

updated 11.2010 


Second's Firsts

Sometimes second, and subsequent, children aren't documented as well as the first, may complain of fewer photographs or feel a tiny bit slighted. I don't know from experience, I've just heard these things might be true... (Andy swears no one ever read to him).
We've been careful to capture some of Tucker's firsts this week. Maybe he'll thank us later.

First bath (Perhaps the Pisces in him will show up at some point, but currently he doesn't seem to be a big fan of water):First read aloud (excluding some random news from CNN online Andy tried soothing him with - apparently the economy is just as distressing to Tuck as it is to most of the rest of us):First bottle (supervised by Colby, of course):JEB


Redefining Perfect

Our family seemed perfect. No white picket fence, and only one child. One who isn't perfect by most standards, but who is perfect to many, especially those who matter. So our family may not have been perfectly perfect, by standard definitions anyway, but perfect to us nonetheless.

Celia's been spending time with her grandparents the past few days, and although she came to visit us in the hospital, and although we very much appreciate this time to bond with Tucker while Celia is well-cared for elsewhere, we've missed her. We've missed the way an occasional smile breaks across her face like the sunrise sets the clouds on fire. The way her yawns, possibly as engaging as the words of an average two-year old, can make us stop whatever we're doing, just to watch and listen. She is perfect.

And Tucker is perfect in his own ways... Right now it's the way he folds up into himself, curling his limbs like a fiddle head fern. And, equally as perfect in the way he stretches out, elongating his body, arching his back, opening his palms, inviting us in.

Our family still seems perfect. It's different now, bigger, and leaning more heavily toward the male end (when you count Colby, and we have to, because he's part of the family and all).
Lots of things have been redefined in our lives lately. But we'll take advantage of these working definitions - of family, of perfection - and tweak them as we go.



Kissing Cousins

Thanks to Uncle Adam for the post title, and thanks to cousin Vanessa for the sweet kisses...
Tucker is one lucky little guy to get to grow up with Vanessa close by. He'd better start storing up energy now, in order to keep up with her. When Vanessa visited earlier this week, she was working on numbers and bouncing balls...
We look forward to Tucker learning lots of things from his older cousin!


Big Sister

It's official. Celia's been a big sister for nine months, but now she's gotten to meet her little sibling...
Clearly, she's thrilled beyond measure.

When I became a big sister, at about the same age that Celia is now, I was equally thrilled. Given a stuffed alligator toy, I insisted "Alligatie sleep with Katie." No dummy, I thought if it didn't eat her that first night, then it wouldn't eat me.

Or maybe I was just really into sharing...



Thomas Everett Betz

March 7th, 2009
11:02 AM
8 pounds, 7 ounces
21 inches

Tuck, Shim, Pirate (a new nickname, thanks again to Aunt Kate, based on the fact that he often looks at things with only one eye open)... No matter what you call him, we're proud to call him our son.



525,600 Minutes

(Note: this post was written previously and scheduled to appear today, on Celia's 2nd birthday, in case we were otherwise engaged with Shim's arrival...)

525,600 minutes, 525,000 moments so dear.

525,600 minutes - how do you measure, measure a year?

In daylights, in sunsets, in midnights, in cups of coffee.

In inches, in miles, in laughter, in strife.
In 525,600 minutes - how do you measure a year in the life?
How about love? How about love? How about love?
Measure in love.

These lyrics, from RENT, remind us that, although it wasn't exactly the kind of year we may have wished for when we helped Celia blow out one candle a year ago, it has been a year full of love, and that's the best way to spend a year, especially when we're not sure how many years we'll have with her...


is on the way! More soon...



Holding Hands

It's a little like touching heaven...




It was about 2 years ago that I was born into motherhood. Writhing on the hospital bed, I thought I might surely die, but instead it was if there were two births that day. Celia made me a mother. She is still making me a mother. I have to dig deep, but there’s the possibility of greatness. And Celia reminds me that greatness is not in me, but comes through me.

I don't often do bold things like shower or eat an entire meal at one sitting or go to the store by myself. But I have better things to do. I get to read books aloud and change diapers, kiss a small forehead and leave projects unfinished.

And, I get to worry. I've gotten a good taste of the primary preoccupation of motherhood – questioning, second-guessing… I bend myself into knots with the constant weighing of pros and cons, often with no discernible “right” answer. I’ve read books about how to be a good mother, and I’ve worried about what others will think. I spend more time with my daughter than authors and neighbors, than anyone, actually, and I often instinctively know what’s right for her. But sometimes I still wonder…

Wonder about her greatness, though, I don't. She was, indisputably, born filled with it.